CIHM 
Microfiche 
Series 
(IVIonographs) 


ICMH 

Collection  de 
microfiches 
(monograph  ies) 


Can.dun  Institut*  for  Hiitorical  Micr<  rtproduction*  /  Institut  Canadian  da  microraproductiona  historiqu 


( 


Technical  and  Bibliographic  Notes  /  Notes  technique  et  bibliographiques 


The  Institute  has  attempted  to  obtain  the  best  original 
copy  available  for  filming.  Features  of  this  copy  which 
may  be  bibliographically  unique,  which  may  alter  any  of 
the  images  in  the  reproduction,  or  which  may 
significantly  change  the  usual  method  of  filming  are 
checked  below. 


0 
□ 

D 

D 
D 
D 

D 

0 

D 

D 

D 


2f 


Coloured  covers  / 
Couverture  de  couleur 

Covers  damaged  / 
Couverture  endommagee 

Covers  restored  and/or  laminated  / 
Couverture  restauree  et/ou  pellicuiee 

Cover  title  missing  /  Le  litre  de  cou-  arture  manque 

Coloured  maps  /  Cartes  geographiques  en  couleur 

Coloured  ink  (i.e.  other  than  blue  or  black)  / 
Encre  de  couleur  (i.e.  autre  que  bleue  ou  noire) 

Coloured  plates  and/or  illustrations  / 
Planches  9t/ou  illustrations  en  couleur 

Bound  witli  other  material  / 
Relie  avec  d'aulres  documents 

Only  edition  available  / 
Seule  edition  disponible 

Tight  binding  may  cause  shadows  or  distortion 
along  interior  margin  /  La  reliure  serree  peut 
causer  de  I'ombre  ou  de  la  distorsion  le  long  de 
la  marge  interieure. 

Blank  leaves  added  during  restorations  may  appear 
within  the  text.  Whenever  possible,  these  have 
been  omitted  from  filming  /  II  se  peut  que  certaines 
pages  blanches  ajoutees  lors  dune  restauration 
apparaissent  dans  le  texte,  mais,  lorsque  cela  etait 
possible,  ces  pages  n'ont  pas  ete  (ilmees. 


L'Institut  a  microfilm^  le  meilleur  examplaire  qu'il  lui  a 
ete  possible  de  se  procurer.  Les  details  de  cet  exem- 
plaire  qui  sont  peut-Stre  uniques  du  point  de  vue  bibli- 
ographique,  qui  peuvent  modifier  une  image  reproduite, 
ou  qui  peuvent  exiger  une  modifications  dans  la  m6th- 
ode  nonmale  iJo  filmage  sont  indiqu6s  ci-dessous. 

I     I      Coloured  pages '  Pages  de  couleur 

I     I      Pages  damaged/ Pages  endommagdes 

I     I      Pages  restored  an*or  laminated  / 
Pages  restaurees  et/ou  pellcul^es 

[^      Pages  discoloured,  stained  or  foxed  / 
Pages  decolorees,  tachetees  ou  piquees 

I     I      Pages  detached  /  Pages  d^tachees 

r^    Shovrthrough  /  Transparence 

I     I      Quality  of  print  varies  / 

— '      Qualite  inegale  de  I'impression 

I     I      Includes  supplementary  material  / 

Comprend  du  materiel  supplementaire 

rn  Pages  wholly  or  partially  obscured  by  errata 
slips,  tissues,  etc.,  have  been  refilmed  to 
ensure  the  best  possible  image  /  Les  pages 
totalement  ou  partiellement  obscurcies  par  un 
feuillet  d'en-ata,  une  pelure,  etc.,  ont  ite  filmees 
a  nouveau  de  fa90n  a  obtenir  la  meilleure 
image  possible. 

r~|  Opposing  pages  with  varying  colouration  or 
discolourations  are  filmed  twice  to  ensure  the 
best  possible  image  /  Les  pages  s'opposant 
ayant  des  colorations  variables  ou  des  decol- 
orations sont  filmees  deux  fois  afin  d'obtenir  la 
meilleur  image  possible. 


Additional  comments  / 
Commentaires  supplementaires: 


Pagination  is  as  follom  :  p.  77-81. 


1 

1 

Thiii 
Ccdo 

lox 

tsm  ii 
cumci 

filmi 
It  lit 

H«tt 
filmi 

litri^ 
«uu 

14  X 

uctio 
jxde 

nf»ti( 
rMuc 

><:lMckMlta«kH>/ 
lion  indiqiu  ci-dessous 

18X 

sx 

2SX 

XX 

■I 

~ 

J 

12X 

1SX 

XX 

24  X 

2a  X 

32  X 

Th«  copy  fllmad  har«  ha*  baan  raproduead  thanks 
to  tha  ganarotity  of: 

Oslar  Library, 

McGill  Univniity, 

Montreal 

Tha  Imagaa  appaaring  hara  ara  tha  bast  quality 
posslbia  eonsidsring  tha  eonditian  and  lagibility 
of  tha  original  copy  and  in  kaaplng  with  tha 
filming  contract  spaeif Icationa. 


Original  copies  in  printed  papar  eevara  ara  fllmsd 
bsglnning  with  tha  front  eovar  and  anding  on 
tha  last  paga  with  a  printad  or  lllustratsd  impraa- 
sion.  or  tha  back  eovar  whan  appropriate.  Ail 
othar  original  copies  ara  filmed  beginning  on  the 
first  page  with  a  printad  or  illustrated  Imprea- 
sion,  and  anding  on  tha  last  page  with  a  printed 
or  illustrated  impression. 


Tie  last  rscorded  frame  on  eech  microfiche 
shell  contain  tha  lymbol  — *■  Imeening  "CON- 
TINUED"), or  the  symbol  ▼  Imeening  "END"), 
whichever  applies. 

Meps,  plates,  charts,  etc.,  mey  be  filmed  st 
different  reduction  ratios.  Thoss  too  large  to  be 
entirely  included  In  one  exposure  are  filmed 
beginning  in  the  upper  left  hend  comer,  left  to 
right  and  top  to  bottom,  as  many  frames  as 
rsquired.  The  fallowing  diegrems  illustrete  the 
method: 


1  2  3 


1  2 

4  5 


L'axamplair*  fllm<  fut  rtproduit  grie*  i  la 
gintrotit*  da: 

Otl«  Library, 

McGill  Uni»tr>itY. 


La«  imagat  luivantaa  ont  M  raproduitas  avac  la 
plua  grand  toin,  compta  tanu  da  la  condition  at 
da  la  nanata  da  I'axamplaira  film*,  at  an 
eonformit*  avac  laa  conditiona  du  contrat  da 
filmaga. 

Laa  axamplalraa  originaux  dont  la  couvartura  an 
papiar  aat  imprini«a  aont  fllmto  an  commandant 
par  la  pramiar  plat  at  an  tarmlnant  aoit  par  la 
darnitra  paga  qui  eomporta  una  amprainta 
d'Impraaalon  ou  d'illuatration.  aoit  par  la  tacond 
plat,  aalon  la  eaa.  Toua  laa  aulraa  axamplairat 
originaux  lont  filmta  an  commandant  par  la 
pramitra  paga  qui  eomporta  una  amprainta 
d'Impraaalon  ou  d'illuatration  at  an  tarmlnant  par 
la  darnitra  paga  qui  eomporta  una  talla 
amprainta. 

Un  daa  aymbolaa  auivanta  apparattra  aur  la 
darniira  imaga  da  ehaqua  microfieha.  aalon  la 
eaa:  la  aymbola  -^  aignifia  "A  SUIVRE".  la 
aymbola  ▼  aignifia  "FIN". 

Laa  eartaa.  planchaa.  Mblaaux.  ate.  pauvant  ttra 
filmta  t  daa  taux  da  rMuetion  difftrantt. 
Loraqua  la  doeumant  aat  trop  grand  pour  ttra 
raproduit  an  un  aaul  cllcM.  11  aat  film*  t  partir 
da  I'anglo  aup4riaur  gaucha,  da  gaucha  i  drolta. 
at  da  haul  an  baa.  an  pranant  la  nombra 
d'imagaa  nicaaaaira.  Laa  diagrammaa  auivanta 
llluatrant  la  mathoda. 


2 

3 

5 

6 

MKROCOPY   ■UOUITION   TBT  CHART 

(ANSI  and  ISO  TEST  CHART  No.  3) 


1.0    ^*^  1^ 


.s    TIPPLED  IM/1GE 


ffif--    yr,£. 


STATISTICS  OF  CONGENITAL  CARDIAC 
DISEASE 


% 


1400  OASES  ANALYZED) 


MAUDE    E    AHUOTT,  H.A„  MI). 


Tub  Jouknal  of  Mbuical  Hi 


IbBAHCK,    VOLUMB    XIX,,   No.    1 

(N«w  Serioa,  VM.  XIV.,  No,  i ).  pp,  7:-Si.  Julv,  igo» 


■'I' 


I 

I 


BOSTON 

MASSACHUSETTS 

U,S.A. 


I 

'5 


» 


'•*'*'»~*»«»«MPM«»»«XH' «f 


ob 


Ill    iiiNIIEMTAr     I\R11TM     li|>t;,\SK. 


Thf  subject  of  congenital  cardiac  disease  is  one  that  lends 
iUelf  well  to  statistical  study,  for  (he  conditions,  being  often 
complex  and  of  recognized  rarity,  are  usually  rep  irted  in 
nnicli  detail.  Moreover,  the  cases  are  so  infrequent  in  any 
one  person's  e.vpcrience  tliat  some  micIi  method  as  this,  of 
makini;  use  of  the  availahle  literature,  must  be  ,id<i|ited  in 
order  to  arrive  at  any  generalizations. 

Tor  another  purpose.  1  have  had  occasion  to  make  a  detailed 
statistical  study  of  some  four  hundred  and  twelve  cardiac 
defects,  A  few  of  these  are  drawn  from  persou.il  experience, 
the  remainder  from  the  literature.  ( Inly  well-authenticated 
cases  with  post-mortem  report  attaclied  have  been  included. 
The  only  exception  to  this  statement  is  formed  by  three  cases 
included  In  the  series  of  patent  ductus  arteriosus  diagnosed 
by  characteristic  physical  signs  and  by  the  X-Rays  and  not 
confirmed  by  post-mortem.  The  results  of  the  analysis  of 
four  hundred  of  these  cases  are  shown  in  the  accompanying 
chart.  This  chart  is  presented  here  merely  as  a  demonstra- 
tion of  the  manner  in  which  these  defects  were  studied,  and 
without  any  intention  of  entering  at  length  into  the  ligures. 
It  represents  a  chart  which  was  originally  printed  for  the 
analysis  of  the  individual  defect,  .i.id  is  here  modified  in  a  few 
particulars  to  admit  of  the  presentation  of  the  total  results 
obtained. 


The  chart  presents  four  main  divisions.  The  First  Division 
includes  the  Classification  of  the  defect.  Number  of  cases 
analyzed,  Age.  and  Sex.  In  the  classification  a  simple 
anatomical    order    h.is    been    followed,   based    also    on    the 

■  Fresenicl  l.efore  ihc  Assod-ii'nn  oJ  i'Lithologlsts  and  Iticlerioiagisis  ai  Ann  .\rl>or 
Micli.,  April  18.  i.»8.      Ke,-,.iv<-.l  for  publk-.-iiion  May  I.  igo8. 


jA  \nii'tn 

pri  liplc",  of  the  develnpmcn*  of  :he  heart,  io  f-.r  as  thcfc  are 
known  Thus,  defects  of  the  cardiac  an-'  aortic  septa  are 
followed  by  transpusition  of  the  arterial  trunks,  due  (accord- 
ing to  Kukitaii^ky)  to  a  deviation  of  t'..e  aortic  septum  ;  and 
this  a^iain  by  pulmonary  and  aortic  stenosis  or  atresia  (some 
cases  of  which  arc  probably  likewise  due  t  >  a  deviation  of  the 
aortic  septum).  The  cahos  of  pulmonary  and  aortic  stenosis 
or  atresia  arc  sub-classified  (foUowinii  Rauchfuss)  according 
to  the  presence  or  absence  of  defects  of  the  intcrauricular  and 
interventricular  septa,  and  this  affords  a  clinical  grouping  of 
much  value,  hv  coarctatiMU  of  the  aorta  the  distinction 
drawn  by  Hountt '  is  observed  between  the  infanliL-  form,  a 
simple  persistence  of  the  isthmus  aortii-,  and  t'lC  typical 
'•  a«tult  t;je"  of  coarctation  in  which  the  aorta  is  obstructed 
or  even  oblitir.»ted  by  a  sharp  constriction  at  or  above  the 
insertion  of  tlif  tluctus. 

Amunv;  the  defeat-*  enumeratcti  in  this  classification  those 
of  clinical  importance  are: 

Defects  of  the  interaurlcular  septum,  2S  cases;  defects  of 
t:.e  interventricular  septum,  40  cases;  cuniplete  defects  of 
the  cardiac  septa  (biloculate  heart,  etc.),  \2  cases;  defects 
of  the  aortic  septum,  14  cases;  transposition  of  the  arterial 
trunks,  44  cases;  pulmonary  stenosis,  75  cases;  pulmonary 
atresia.  23  cases;  tricuspid  stenosis,  2  cases;  tricuspid 
atresia,  <j  cases;  patent  ductus  arteriosus,  2^  cases  ;  coarcta- 
tion of  the  aorta,  }}  cases;  hypoplasia  of  the  aorta.  2  cases. 
iJndcr  -Nge  "  are  three  columns  in  which  the  maximum, 
minimum,  and  mean  a^jes  of  the  cases  in  each  grcup  are  cal- 
culated. 


The    Seconj    Divisii.n  the   chart  includes  tht-""    Post- 

mortem Findings  of  especial  importance  in  cardiac  defects. 
Here  are  noted  the  condition  of  the  fetal  passages,  whether 
closed  or  patent,  the  pre-ence  of  hypoplasia  or  dilatation  of 
the  pulmonary  artery  or  the  aorta,  the  existence  of  a  col- 
lateral ci^-culation  (important  in  coarctation  of  'he  aorta), 
the  incid^'nce  of  arterial  disease,  of  acute  endocarditis,  and  of 

•  Honiift.     R.-vm:  dc  Mi'^ilicini-,  1903. 


slAIIMh 


i>t 


'MJKMIAI     <    \Uhl\'      hl^l.A-i  . 


79 


chronic  valvu'.ir  ilisc.isc,  tlu-  prcseiut'  of  hyp -rtropliy  aiu! 
(lil.ilation  of  Uu'  •litfcrciu  cli.inihcrt  nt  t)u-  hort,  aiiil  \d%i\y 
the  cxistcnci;  uf  n-fsucialctl  anoiitahcs  in  tlie  heart,  vesscN,  or 
cKcwhcri.', 

The  Ihinl  1  visi  in  potc;  points  of  cUnic.i!  interest, Mich  as 
the  prt'sciicc  of  "(iiuntions  having;  .in  <-tiol";;ical  luMriny  on 
ihc  f.ituily  iiistor)'.  iml  in  the  p'Ttuinl  liistttry  tlie  iiici- 
lience  of  ihcum.itism,  puhiiunary  tiilierciilosis.  or  congenita! 
syphilis,  am!  tlie  pmportion  of  l  '.cs  recu/cnnt;  from  the 
acute  infectious  fevers  (which  cyan  >tic  patients  are  sail!  to 
pass  throut;h  *el!),  L'culer  special  symptoms  arc  cr)lunins 
for  cyanosis  in  its  ditVerciit  (let,'rees,  clubbing  of  the  finj^ern, 
tlyspnea,  djspncic  attacks  and  delayed  development.  I'hysical 
sitjns  may  be  vascular  or  cardiac,  .iml  aniun^,'  the  tatter  the 
occurrence  ))f  visible  [luK.ition.  precordial  bulj^iiij;,  llirill. 
increased  dulness,  accentuation  ol  the  heart  sounds,  ,inJ 
the  existence  of  murmurs,  presystolic,  systolic,  diastolic. 
Continuous,  double  (i.e.,  systolic  and  disastcdic  in  rhythm), 
or  indefinitely  stated,  arc  noted.  I-'inally.  under  causes  of 
death  we  find  the  defect  itself  provinj*  fatal  sudtlenly  or  by 
failing  com|)ensation,  or  a  termination  by  bronchn-pncumonia, 
cerebral  complications  or  the  acute  infectious  fevers. 


The  Fourth  Division  of  the  chart,  that  of  Relative  Fre- 
i|ucncy,  3  of  the  j;reati\st  importance.  Cardiac  anomalies 
are  so  of:en  complicated  that  the  number  of  times  a  {^iven 
defect  occurs  alone  or  as  the  primary  condition  by  no  means 
represents  its  total  incidcur-;  in  the  four  hundrei!  cases.  In 
this  division  there  are,  therefore,  ihree  columns.  In  the  first 
of  these  stands  "  the  number  of  cases  classified  as  the  pri- 
mary lesion,"  the  figures  of  wh'ch  are  identical  wiih  those  at 
the  beyinninti  of  the  chart  showinj^  '■  the  number  of  cases 
analyzed"  in  each  group.  The  sum  of  the  figures  in  this 
column  is  the  four  hundred  cases  analyzed.  In  the  next 
column  stands  the  number  of  cases  in  each  group  in  which 
the  defer  occurs  complicating;  other  conditions,  and  this 
with   the  number  of  cases  classified  as  the   primary   lesion 


So 


l[ivi-<t  the  tuul   inchUnci-  of  the  itcrcct 
ill  the  liiit  column  of  the  chart. 


which  is  ihi.i  nhovvn 


The  result  of  thi-t  .tiiiilvsi**  brinn*  out  some  rirmarkiihlc 
f-ictn,  ncvcriil  of  which  art  at  v;ir..»nce  with  .iccL-pUil  iiii  .i-^. 
The  fnlluwinvi  (»»tnt-t  arc  ..f  rspccial  inti-rt-Ht : 

1 .  The  frei|iiLncy  uf  tk-ft-cts  of  thi-  intcrvt  utricular 
septum.  — While  rL-lativcly  rare  altujc  ( lliirty-twu  difictn  at 
the  base  amoiiK  the  four  huiulrcd),  in  combinati>>u  witli 
iithcr  comlition'^  thjs  is  seen  (o  be  the  moil  common  of  nil 
cardiac  anomalies  {<tne  hundrcil  and  fiTty  nine  annmt;  the 
four  hundred  cases) ;  next  in  frc(iu<-ncy  comes  patent  fora- 
men ovale,  under  which  arc  includeil  only  cases  of  true 
patency,  nut  simply  a  valvular  or  slit-like  comlition,  with  one 
hundred  and  thirty  four  cases,  and  then  patent  ductus  arte- 
riosus with  one  hundred  and  six.  The  frequency  of  trans- 
position of  the  arterial  trunks  (forty-six  cases)  and  of 
pulmonary  stenosis  with  defect  of  the  interventricular  sep- 
tum (seventy-three  cases)  is  noteworthy,  while  pulmonary 
stenosis  with  closed  interventricular  septum  is  relatively 
infrequent   (seventeen  cases). 

2.  The  duration  of  life  is  seen  to  be  relatively  lont;  in 
uncomplicated  defects  of  the  interauricut.ir  septum,  patent 
ductus  arteriosus,  coarctation  of  the  aorta,  and  pulmonary 
stenosis  witli  closed  interventricular  septum.  In  pulmonary 
stenosis  with  defect  of  this  septum  the  iluration  of  life  is 
seen  to  be  much  shorter. 

3.  I'atcncy  of  the  ductus  arteriosus  is  seen  to  be  rare  in 
pulmonary  stenosis,  though  very  frequent  in  [lulmonary 
atresia. 

4.  The  riiiht  chambers  chief!)-  are  hypertrophied  and 
dilated  in  defects  of  the  inierauricular  septum,  transposition 
of  the  arterial  trunks,  pulmonary  stenosis  and  atresia.  Both 
chambers,  but  chiefly  the  rijjht.  arc  enlar^jed  in  defects  of 
the  interventricular  septum  and  patent  ductus  arteriosus,  the 
left  ventricle  chiefly  in  coarctation  of  the  aorta. 

3.     Acutt  endocarditis  is  seen  to  be  relatively  common  in 


if 


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lONCENlTM,   CAKl.lAC    IIISKASE. 


8l 


6.     Cyanosis  was   absent   i„   most    of  the  defects  of  the 

"'",  A  tnoderate  degree  of  eyanosis  was  fairly  common 
■n  defects  of  the  interventricular  septum,  a  mar  Jd  ." 
only  three  cases.  Marked  cyanosis  was  seen  chieHy  n'tran 
pos,^„  o  the  arterial  trunks,  pulmonary  stenosis  Jit  ;  . 
of  he  ,merve„tr,cular  septum,  pulmonary  and  tricuspid 
atresia.     Cyanosis    w.-,s    usually    slight  or    ab.sent    in  patent 

uc  us  artenosus  and  in  coarctation  of  the  aorta  of  the'  it 
t,|.e.     I,  sx  cases  of  defect  of  the  interauricular  and  in  four 

•  terminal  •  ■"'"-"'-">-   -P<-..  .1-  cyanosis   w 

terminal     appear.ni;  only  in  the  last  few  weeks  of  life 

veninV  ,  ""•'  ^"■■''"""  '■"  "^'"'"  ''••■'■"^'^  "f  the  inter- 

ventricular  septum  at  the  base,  and  in  pulmonary  ste„o"s 
w.. .  closed  ,nterve„tricular  septum,  or  with  defect  of'^tl  e  i^e 
ven tncular  septum  and  patent  foramen  ovale.     A        I    was 
rela  ,vely  rare  in  pulmonary  stenosis  with  defect  of  t  ,     n  er 
ventr.cular  septum  and  closed  foramen  ovale 

8      In  the  great  majority  of  cardiac  defects  the  murmur 
when  present,  was  systolic  in  rhythm. 

9.  In  some  cases  of  pulmonary  stenosis  the  pulmonary 
second  sound  was  accentuated.  mionary 

These  are  not  all  the  conclusions  to  be  drawn  from  a  study 
of  .h,s  analytical  table;  they  are  sufficient,  however,  to  show 
the  value  of  a  careful  and  detailed  tabulation  of  the  dati 
Z[  by  different  observers  in  arriving  at  genera  dedut 
..ons,  such  as  could  not  legitimately  be  drawn  fron,  the  facts 
■n  the  experience  of  any  single  worker. 


